Chapter 12. Diagnosis and Treatment of Primary (Idiopathic) Dystonia

  1. Nils Erik Gilhus MD, PhD Professor11,
  2. Michael P. Barnes MD, FRCP Professor of Neurological Rehabilitation Medical Director12 and
  3. Michael Brainin MD Professor13
  1. A. Albanese1,2,
  2. F. Asmus3,
  3. A. Berardelli4,
  4. K. Bhatia5,
  5. A. E. Elia1,
  6. B. Elibol6,
  7. G. Filippini1,
  8. T. Gasser2,
  9. J. K. Krauss7,
  10. N. Nardocci1,
  11. A. Newton8,
  12. J. Valls-Solé9 and
  13. M. Vidailhet10

Published Online: 6 SEP 2010

DOI: 10.1002/9781444328394.ch12

European Handbook of Neurological Management, Second Edition, Volume 1, Second Edition

European Handbook of Neurological Management, Second Edition, Volume 1, Second Edition

How to Cite

Gilhus, N. E., Barnes, M. P. and Brainin, M. (2010) Diagnosis and Treatment of Primary (Idiopathic) Dystonia, in European Handbook of Neurological Management, Second Edition, Volume 1, Second Edition, Wiley-Blackwell, Oxford, UK. doi: 10.1002/9781444328394.ch12

Author Information

  1. 11

    Department of Clinical Medicine, University of Bergen and Department of Neurology, Haukeland University Hospital, Bergen, Norway

  2. 12

    University of Newcastle, Hunters Moor Neurorehabilitation Ltd, Newcastle upon Tyne, UK

  3. 13

    Department of Clinical Medicine and Prevention, and Center for Clinical Neurosciences, Donau-Universität Krems, Department of Neurology, Landesklinikum Donauregion Tulln Tulln, Austria

Author Information

  1. 1

    Istituto Neurologico Carlo Besta, Milan, Italy

  2. 2

    Università Cattolica del Sacro Cuore, Milan, Italy

  3. 3

    Hertie-Institute for Clinical Brain Research, University of Tubingen, Germany

  4. 4

    Università degli Studi La Sapienza, Rome, Italy

  5. 5

    Institute of Neurology, University College London, Queen Square, London, UK

  6. 6

    Hacettepe University Hospitals, Ankara, Turkey

  7. 7

    Medical School Hannover, MHH, Germany

  8. 8

    European Dystonia Federation, Brussels, Belgium

  9. 9

    Hospital Clínic, Barcelona, Spain

  10. 10

    Pôle des Maladies du Système Nerveux, Fédération de Neurologie, Paris, France

Publication History

  1. Published Online: 6 SEP 2010
  2. Published Print: 15 OCT 2010

ISBN Information

Print ISBN: 9781405185332

Online ISBN: 9781444328394



  • diagnosis and treatment of primary (idiopathic) dystonia;
  • dystonia, characterized by sustained muscle contractions - causing repetitive twisting movements or abnormal postures;
  • classification of dystonia - based on three axes;
  • use of genetic test in diagnosis and counselling;
  • DYT1 mutations, most important genetic cause - of early onset PPD worldwide;
  • most common form, DRD linked to GCH1 (GTPcyclohydrolase I) gene;
  • DYT12, rapid- onset dystonia- parkinsonism (RDP) - mutated gene, being ATP1A3;
  • brain imaging in diagnosis of dystonia;
  • Botulinum toxin treatment, continuing to be first choice treatment - for most types of focal dystonia;
  • long-term electrical stimulation of globus pallidus internus (GPi) - now established as an effective treatment for various types of dystonia


This chapter contains sections titled:

  • Objectives

  • Background

  • Search strategy

  • Method for reaching consensus

  • Results

  • Medical treatments

  • Neurosurgical procedures

  • Physical therapy and rehabilitation

  • References